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Hyperammonaemia in children can lead to grave consequences in the form of cerebral oedema, severe neurological impairment and even death. In infants and children, common causes of hyperammonaemia include urea cycle disorders or organic acidaemias. Few studies have assessed the role of extracorporeal therapies in the management of hyperammonaemia in neonates and children. Moreover, consensus guidelines are lacking for the use of non-kidney replacement therapy (NKRT) and kidney replacement therapies (KRTs, including peritoneal dialysis, continuous KRT, haemodialysis and hybrid therapy) to manage hyperammonaemia in neonates and children. Prompt treatment with KRT and/or NKRT, the choice of which depends on the ammonia concentrations and presenting symptoms of the patient, is crucial. This expert Consensus Statement presents recommendations for the management of hyperammonaemia requiring KRT in paediatric populations. Additional studies are required to strengthen these recommendations.

Background:Endothelins (ET) are a family of peptides that act as potent vasoconstrictors and pro-fibrotic growth factors. ET-1 is integral to renal and cardiovascular pathophysiology and exerts effects via autocrine, paracrine and endocrine signaling pathways tied to regulation of aldosterone, catecholamines, and angiotensin. In the kidney, ET-1 is critical to maintaining renal perfusion and controls glomerular arteriole tone and hemodynamics. It is hypothesized that ET-1 influences the progression of chronic kidney disease (CKD), and the objective of this review is to discuss the pathophysiology, and role of ET and endothelin receptor antagonists (ERAs) in CKD. Summary:The use of ERAs in hypertensive nephropathy has the potential to decrease proteinuria, and in diabetic nephropathy has the potential to restore glycocalyx thickness, also decreasing proteinuria. Focal segmental glomerular sclerosis has no specific Food and Drug Administration-approved therapy currently, however, ERAs show promise in decreasing proteinuria and slowing tissue damage. ET-1 is a potential biomarker for autosomal dominant polycystic kidney disease progression and so it is thought that ERAs may be of some therapeutic benefit. Key Messages:Multiple studies have shown the utility of ERAs in CKD. These agents have shown to reduce blood pressure, proteinuria, and arterial stiffness. However, more clinical trials are needed, and the results of active or recently concluded studies are eagerly awaited.

Despite a long-term focus on passenger vehicle safety, there are still 38,000 vehicle-related fatalities annually. Some are the result of failure to maintain safety components of vehicles, such as brakes, tires, or headlights. Following NHTSA guidelines, 18 states have implemented periodic safety inspection programs in which certified inspectors assess components, and owners are required to repair or replace deficient components. In the case of tires, when the tread depth falls to 2/32 in., its stopping distance becomes excessive. Thus, this tread depth level was built into the safety inspection thresholds for tires. One challenge is that, in an annual vehicle inspection, if a tire passes at a level of 3/32 in., it may fall below the safe (2/32 in.) threshold soon after inspection. In an era of higher vehicle miles traveled (VMT) and reduced attention to maintenance, perhaps the thresholds set for safety inspections should be higher than the “safe level” to provide a buffer. Using 6 million safety inspection records from Pennsylvania from 2006 to 2016 we calculated tread depth deterioration and annual VMT at the vehicle level. We estimated the “percent of vehicles at risk of having unsafe tires before the next inspection” (using the 2/32 in. threshold) to be about 30%. We also estimated how the percent of at-risk vehicles decreases as the inspection thresholds are raised, and found an attractive threshold at about 5/32 in. for which the percent of at-risk vehicles would be very low. Such changes could further reduce fatal and non-fatal accidents.

Background: Patient-Centered Medical Home (PCMH) places patients at the center of care and actively considers patients’ goals, preferences and skill levels in caring for their disease while developing a care plan. We implemented a quality improvement (QI) project to improve internal medicine resident goal-setting activity with patients for chronic disease self-management.